Kognitive Einschränkungen bei erwachsenen Patienten mit Neurofibromatose Typ 1

Uttner, Ingo; Wahlländer-Danek, U.; Danek, Barbara A.

doi:10.1055/s-2003-37759

Cited by 10 publications

(1 citation statement)

References 15 publications

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“…With only a few studies of cognition in adults with NF1, 20 there is a scarce body of knowledge regarding this population. Reviewing the literature on NF1 and ADHD in adults, we found only one report of IQ and attention profiles associated with this comorbidity and no reports of the cognitive progression of both disorders.…”

Section: Introductionmentioning

confidence: 99%

Neurofibromatosis type 1 and attention deficit hyperactivity disorder: a case study and literature review

Miguel¹,

Chaim‐Avancini²,

Silva³

et al. 2015

NDT

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BackgroundThe cognitive profile of children with neurofibromatosis type 1 (NF1) and attention deficit hyperactivity disorder (ADHD) has been well characterized, but few studies have evaluated the cognitive abilities of adults with NF1 and ADHD.ObjectivesWe investigated 1) the cognitive profile of an adult patient with NF1 and inattention problems, 2) changes in his cognition after 14 months of follow-up, and 3) whether the patient exhibited comorbid NF1 and ADHD or secondary ADHD-like symptoms.MethodsWe administered neuropsychological tests of executive function, attention, verbal and visual memory, visuospatial function, and language during two evaluations separated by 14 months.ResultsWe found no changes in sustained attention, language, or verbal memory. Visual memory, verbal learning, selective attention inhibitory control, and problem solving declined over time, whereas visual search, psychomotor speed, visuospatial function, and mental flexibility improved.ConclusionOur patient exhibited a cognitive profile characteristic of both NF1 and ADHD, leading to the hypothesis that the patient had comorbid ADHD instead of secondary ADHD-like symptoms. More studies are necessary to characterize the cognition of patients with NF1 and ADHD.

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Section: Introductionmentioning

confidence: 99%

Neurofibromatosis type 1 and attention deficit hyperactivity disorder: a case study and literature review

Miguel¹,

Chaim‐Avancini²,

Silva³

et al. 2015

NDT

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Adolescents and young adults with neurofibromatosis type 1: A descriptive study of adaptive functioning

Struemph

Watts

Wolters

et al. 2021

American J of Med Genetics Pt A

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Neurofibromatosis type 1 (NF1) is a genetic syndrome affecting about 1 in 3500 individuals; many of those affected have plexiform neurofibroma (pNF) tumors and associated symptoms and complications. Furthermore, learning and attention problems, as well as deficits in adaptive functioning, are common, often beginning in early childhood. This study aimed to describe adaptive functioning and to examine relationships between adaptive functioning and cognitive and academic variables and level of independence among adolescents and young adults (AYA) with NF1 and pNF tumors. Fifty-five AYA aged 16-31 years participated in a series of neuropsychological evaluations while parents completed the Vineland Adaptive Behavior Scales (VABS-II) as part of a larger natural history study. Over one-third (35%) of AYA were neither in school nor employed. Mean VABS-II daily living and socialization scores were low average while mean Verbal and Performance IQ scores were average. VABS-II scores were positively correlated with processing speed, executive functioning, and working memory scores. Verbal IQ was the only significant predictor of work/school status. Identification of the correlates and predictors of adaptive functioning and life achievement can help guide healthcare providers with the early identification of risk factors and possible areas for intervention. K E Y W O R D S adaptive functioning, adolescents and young adults (AYA), NF1 1 | INTRODUCTION Research suggests that early adaptive skills in young children with neurofibromatosis type 1 (NF1) are lower than same-aged peers (Klein-Tasman et al, 2013). Adaptive behavior is commonly defined as how people meet their daily needs and navigate the expectations in their day-to-day interactions. Areas of adaptive functioning include communication, daily living skills, and socialization (Sparrow, Cicchetti, & Balla, 2005). For individuals diagnosed with NF1, these deficits continue (Cohen et al., 2020;Payne et al., 2021) and in one study worsen (Glad et al., 2020) throughout childhood and adolescence. These deficits have even been documented in the context of average cognitive abilities (Eby, Griffith, Gutmann, & Morris, 2019). Glad et al. (2020) found executive functioning in early childhood to be predictive of adaptive functioning in the school-age period.NF1 is a genetic disorder that occurs in approximately 1 out of every 2500-3500 births and is typically diagnosed in early childhood (Evans et al., 2010;Tonsgard, 2006). NF1 causes a wide variety of physical, psychosocial, and cognitive impairments, all of which may contribute to challenges in adaptive functioning, particularly during the years of transition from adolescence to young adulthood. Physical [Correction added on 24 December 2021, after first online publication: The blinded text has been replaced with "National Cancer Institute" at all occurrences in the article.]

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Neurogenetik und die Genetik des Verhaltens

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Kognitive Einschränkungen bei erwachsenen Patienten mit Neurofibromatose Typ 1

Cited by 10 publications

References 15 publications

Neurofibromatosis type 1 and attention deficit hyperactivity disorder: a case study and literature review

Neurofibromatosis type 1 and attention deficit hyperactivity disorder: a case study and literature review

Adolescents and young adults with neurofibromatosis type 1: A descriptive study of adaptive functioning

Neurogenetik und die Genetik des Verhaltens

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